Clinical outcomes and protocol for the management of isolated congenital diaphragmatic hernia based on our prenatal risk stratification system.

ABSTRACT

BACKGROUND/PURPOSE: The aim of this study was to evaluate our prenatal risk stratification system for risk-adjusted management in fetuses with isolated congenital diaphragmatic hernia (CDH). METHODS: Ninety-four infants prenatally diagnosed with isolated CDH treated between 1998 and 2017 at our institution were included in this retrospective single-center cohort study. RESULTS: The patients were prenatally classified into four risk groups Group A (n = 54), which consisted of infants with neither liver-up nor a contralateral lung-to-thorax transverse area (L/T) ratio <0.08. The infants in group A were divided into two subgroups Group A-1 (n = 24) consisted of mild conditions; and Group A-2 (n = 30) consisted of severe conditions; Group B (n = 23), which consisted of infants with either liver-up or L/T ratio <0.08; and Group C (n = 17), which consisted of infants with both liver-up and L/T ratio <0.08. The rates of survival to discharge in Groups A-1, A-2, B, and C were 100.0%, 100.0%, 87.0%, and 58.8%, respectively. The rates of intact discharge were 91.7%, 90.0%, 52.1%, and 23.5%, respectively. CONCLUSIONS: Our prenatal risk stratification system demonstrated a significant difference in the severity of postnatal status and clinical outcomes between the groups. STUDY TYPE Case Series, Retrospective Review. LEVELS OF EVIDENCE LEVEL IV.